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| Year : 2012 | Volume
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| Issue : 2 | Page : 343-345 |
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| Diffuse proliferative crescentic lupus nephritis with normal renal function |
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Vibhanshu Gupta1, Sandeep Sachdeva2, Anwar Salamat Khan1, Shahzad Faisul Haque1
1 Department of Nephrology, Jawaharlal Nehru Medical College (JNMC), Aligarh Muslim University (AMU), Aligarh, India
2 Department of Pediatrics, Jawaharlal Nehru Medical College (JNMC), Aligarh Muslim University (AMU), Aligarh, India
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| Date of Web Publication | 28-Feb-2012 |
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 Abstract | | |
Crescentic nephritis can be a manifestation of Lupus Nephritis. It usually presents with rapid deterioration of renal functions over days to weeks with an active urinary sediment and confers a poor prognosis. Nephrotic range proteinuria is rare in crescentic nephritis. We present an interesting case of (crescentic) Diffuse Proliferative Lupus Nephritis (DPLN) with remarkably preserved renal function and nephrotic range proteinuria. Also noteworthy was the patient's excellent response to immunosuppressive therapy despite the high frequency of cellular crescents on biopsy.
How to cite this article:
Gupta V, Sachdeva S, Khan AS, Haque SF. Diffuse proliferative crescentic lupus nephritis with normal renal function. Saudi J Kidney Dis Transpl 2012;23:343-5 |
How to cite this URL:
Gupta V, Sachdeva S, Khan AS, Haque SF. Diffuse proliferative crescentic lupus nephritis with normal renal function. Saudi J Kidney Dis Transpl [serial online] 2012 [cited 2021 Mar 1];23:343-5. Available from: https://www.sjkdt.org/text.asp?2012/23/2/343/93171 |
| Introduction | |  |
Though Crescentic Glomerulonephritis is a characteristic of ANCA positive vasculitic syndromes, it may less commonly be a manifestation of Lupus Nephritis, limited to mainly ISN class IV and III. [1] While ISN class IV Diffuse Proliferative Lupus Nephritis (DPLN) itself carries a poor prognosis if timely intervention is not carried out, the formation of crescents further warrants rapid deterioration of renal function and a very poor outcome. [1],[2] However, we present a case of class IV Crescentic Lupus Nephritis with nephrotic range proteinuria and preserved renal function who responded remarkably well to treatment.
| Case Report | | |
An 18-year-old young girl presented with complaints of progressive swelling over body since previous 3-4 weeks, along with decreased urinary output. There was no history of hema-turia, sore throat, skin infection, or any other significant past events. On examination, she was hypertensive with a blood pressure of 160/100 mmHg and had anasarca. Her urine examination showed 4+ albumin with presence of 4-5 erythrocytes and 10-12 leukocytes/High Power Field with numerous erythrocytes and leukocyte casts. Her renal functions showed a blood urea nitrogen of 15 mg% (normal range: 10-20 mg%) and serum creatinine (SCr) of 1.2 mg% (normal: <1.5 mg%). The total urinary proteins (TUP) were 6 g/24 hours, total serum proteins were 3.9 g% (normal range: 5.5-8.5 g%), serum albumin was 1.6 g% (normal range: 3.5-5.5 g%), and serum globulin was 2.3 g% (normal range: 2.5-3.5 g%). Serum lipid profile revealed low-density lipoprotein (LDL) cholesterol 301 mg% and triglycerides 246 mg%. Immunological studies showed anti-streptolysin O negative, antinuclear antibody positive, and anti-ds DNA positive (140 IU/mL titer). Appropriate diuretic therapy was administered and a renal biopsy was performed when edema subsided. The biopsy report showed diffuse endo-capillary proliferation with 10 out of 16 glomeruli (60%) showing partial and circumferential cellular crescents and one glome-rulus with fibrocellular crescent with moderate chronic tubulointerstitial nephritis and patchy tubular atrophy [Figure 1]. A diagnosis of Class IV crescentic DPLN was made and patient was started on monthly pulse cyclophosphamide and oral methylprednisolone 1 mg/kg body weight. The patient responded with reduction in proteinuria to 0.5 g/day in five months (till date) and resolution of edema and normalization of urinary sediment though hypertension persisted.  | Figure 1: Slide showing two glomeruli: one in the center, showing circumferential crescent formation and the other one in the periphery (11 o'clock position) with crescent formation.
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| Discussion | | |
Nephrotic range proteinuria occurs rarely in crescentic nephritis [3] which was a characteristic feature of this case. Also, several researchers have shown that class IV Lupus with extensive crescent formation carries a grave prognosis and most patients have gross derangement in renal functions. The percentage of glomeruli that show crescents usually correlates with the severity of renal failure in their studies. [4],[5],[6],[7],[8] This was in contrast with our observation of a relatively preserved renal function in spite of more than 60% crescents on biopsy. However, lately, research has shown that in almost all the crescentic nephritides, the extent of renal failure, and therefore the prognosis, are largely a function of the chronicity indices (i.e. tubulointerstitial scarring, followed in order by glomerular sclerosis and crescentic fibrosis), and the number of normal glomeruli, rather than any measure of crescentic glomeruli alone. [3],[8] It is, therefore, a possibility that the presence of only a moderate chronic tubulointerstitial nephritis resulted in a relatively well-preserved renal function in our case. Also, since all the crescents were active cellular crescents, timely initiation of immunosuppression must have resulted in regression of proteinuria and resolution of the disease process.
We infer that active cellular crescentic nephritis can respond well to timely immunosup-pression, irrespective of the number of cre-cents. Renal failure correlates better with the presence of markers of chronicity, i.e. glome-rular sclerosis, fibrous crescents, tubular atrophy, and interstitial fibrosis, which were minimal in this case. [8],[9]
| References | | |
| 1. | Leaker B, Fairley KF, Dowling J, Kincaid-Smith P. Lupus nephritis: clinical and pathological correlation. Q J Med 1987;62:163-79. 
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| 2. | Sumethkul V, Chalermsanyakorn P, Changsiri-kulchai S, Radinahamed P. Lupus nephritis: a challenging cause of rapidly progressive cres-centic glomerulonephritis. Lupus 2000;9:424- 8.
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| 3. | Levy J, Pusey-Charles D. Crescentic nephritis. Oxford Textbook Of Clinical Nephrology 3 rd ed.(Vol. I),315.
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| 4. | Couser WG. Rapidly progressive glomerulo-nephritis: Classification, Pathogenetic mechanisms and therapy. Am J Kidney Dis 1988;11 (6):449-64.
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| 5. | Whitworth JA, Morel-Maroger L, Mignon F, Richet G. The significance of extracapillary proliferation. Clinicopathological review of 60 patients. Nephron 1976;16:1-19.
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| 6. | Beirne GJ, Wagnild JP, Zimmerman SW, Macken PD, Burkholder PM. Idiopathic crescentic glo-merulonephritis. Medicine 1977;56:349-81.
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| 7. | Morrin, PA, Hinglais N, Nabarra B, Kreis H. Rapidly progressive glomerulonephritis. A clinical and pathologic study. Am J Med 1978; 65:446-60.
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| 8. | Austin HA 3d, Muenz LR, Joyce KM, Anto-novych TT, Balow JE. Diffuse proliferative lupus nephritis: identification of specific pathologic features affecting renal outcome. Kidney Int 1984;25(4):689-95.
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| 9. | Sasatomi Y, Kiyoshi Y, Takabeyashi S. A clinical and pathological study on the characteristics and factors influencing the prognosis of crescentic glomerulonephritis using a cluster analysis. Pathol Int 1999;49(9):781-5.
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Correspondence Address:
Sandeep Sachdeva
Department of Pediatrics, Jawaharlal Nehru Medical College (JNMC), Aligarh Muslim University (AMU), Aligarh
India
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PMID: 22382232 
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